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Cystic Fibrosis - Crossref

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Last Updated: 23 June 2022

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Is the type of chronic pulmonary infection a determinant of lung function outcomes in adult patients with cystic fibrosis?

The leading causes of the high mortality of cystic fibrosis patients are lung function abnormalities. It is not yet clear if Burkholderia cepacia infection causes more widespread loss of lung function than Pseudomonas aeruginosa infections. Our main aim was to determine the lung function of adult CF patients with various chronic pulmonary infections. In patients infected with B. cepacia complex, FVC was noticeably more affected; in contrast, FEF25-75% was significantly greater in patients with P. aeruginosa virus infections. Patients with persistent P. aeruginosa disease and chronic B. cepacia complex infection were primarily homozygous for u00ceu201dF508, respectively. Although chronic B. cepacia complex infection leads to less frequent lung volume reduction, chronic P. aeruginosa infection causes a more prominent obstruction of small airways. Our results also show that u00ce-homozygous patients are more susceptible to persistent P. aeruginosa infection.

Source link: https://doi.org/10.4081/monaldi.2012.145


Ventilatory muscle strength in cystic fibrosis patients: a literature review

Changes in the respiratory muscle endurance tests may arise as a result of cystic fibrosis patients' change in the ventilatory mechanics. Aim: To analyze, trough a literature review, the respiratory muscle strength in CF patients's respiratory muscle endurance. The overwhelming majority of the studies, 74%, showed average or above average respiratory muscle strength, while the remaining 28 percent showed diminished or near-normal values. The results of several studies in this cohort contradicting results regarding respiratory muscle strength in these patients' are mixed. However, the majority of the studies indicate that CF patients had maximum respiratory pressures above or above predicted values.

Source link: https://doi.org/10.4081/monaldi.2012.147


A Clinical and Molecular Survey of 62 Cystic Fibrosis Patients from Umbria (Central Italy) Disclosing a High Frequency (2.4%) of the 2184insA Allele: Implications for Screening

Genetic testing procedures and diagnosis of cystic fibrosis can be difficult due to the presence of extreme allelic heterogeneity and the difficult clinical interpretation of rare variants. Since Umbria was excluded from the initial survey of Italian CF patients due to the poor number of chromosomes tested, we've conducted a comprehensive retrospective clinical and molecular analysis of 62 CF patients originating from this region, we'll have continued.

Source link: https://doi.org/10.1159/000450849


Population-Based Genetic Screening for Cystic Fibrosis: Attitudes and Outcomes

In Victoria, Australia, a population-based cystic fibrosis carrier screening service was introduced in 2006, and was extended to couples planning a pregnancy or early pregnancy for a fee. The aim of this survey was to determine people who were undergoing screening to determine their screening behaviors. The questionnaire was returned by forty-seven airlines and 65 non-carriers. The main reasons for choosing screening were the recognition of CF as a chronic disease and a physicianu2019s recommendation. Correctly recalled their carrier status and the danger of having a child with CF, while 3 non-carriers were unsure of their carrier status and 12 incorrectly reported their residual danger. More often than non-carriers, Carriers answered the knowledge questions correctly. There was no difference in fear between carriers and non-carriers. The majority of carriers notified their families of the increased danger of being a carrier. Participants' response to carrier screening for CF was generally positive, according to we.

Source link: https://doi.org/10.1159/000276544


Assessing the Potential Success of Cystic Fibrosis Carrier Screening: Lessons Learned from Tay-Sachs Disease and β-Thalassemia

Unlike TSD and u03b2-thalassemia, the aim of current CF carrier screening policies is informed reproductive decision-making, without the intention of reducing disease prevalence. Because of its unique role, CF carrier screening will require different measures of success than those used for TSD and u03b2-thalassemia carrier screening, and a consensus on the benefits or success of CF carrier screening may be impossible to achieve.

Source link: https://doi.org/10.1159/000253122


Therapeutic Options for Hydrating Airway Mucus in Cystic Fibrosis

BACKGROUND: Genetic mutations in the CF transmembrane conductance regulator gene result in reduced chloride efflux from ciliated airway epithelial cells in cystic fibrosis. This results in a decrease in the periciliary liquid depth of the airway surface liquid as a result of reduced water efflux. The dehydration of mucociliary clearance decreases mucociliary clearance, leading to airway obstruction with mucus plug formation. MCC rises as a result of hydrating mucus. Direct hydration can be achieved by direct hydration, using CFTR modulators to correct dysfunctional CFTR, or it can be achieved pharmacologically. Other CFTR correctors are also showing pre-clinical evidence.

Source link: https://doi.org/10.1159/000377638


The Use of Artificial Sputum Media to Enhance Investigation and Subsequent Treatment of Cystic Fibrosis Bacterial Infections

Mutations in the CF transmembrane conductance regulator protein reduce ionic exchange in the lung, resulting in thicker mucus, which hinders mucociliary function, airway inflammation, and infection. A long-term aim of in vitro study into CF sputum infections is to better understand bacterial growth and infection pathways. The introduction of host cell layers brings artificial sputum medium from a useful in vitro method for determining bacterial functions used in CF lung to uses in the manufacture of antimicrobial treatment regimens and analysis of interactions at the epithelial cell surface and interior. In 1997, the first iteration of artificial sputum media was introduced. The ability to standardize media composition according to experimental specifications is a major advantage of artificial sputum media.

Source link: https://doi.org/10.3390/microorganisms10071269


Lung Pathology in Response to Repeated Exposure to Staphylococcus aureus in Congenic Residual Function Cystic Fibrosis Mice Does Not Increase in Response to Decreased CFTR Levels or Increased Bacterial Load

Using mouse models of cystic fibrosis, congenic or inbred strains, it was determined that defects in murine Cftr increased the sensitivity to Staphylococcus autr. Methods: We review histopathological studies of CF mice repeatedly exposed by aerosolization to a CF isolate of S. austria, all congenic on the C57Bl6/N background. In reaction to repeated exposure to the most common early CF lung pathogen S. aurus, mice congenic on the C57Bl/6 background have significantly more severe lung disease than non-CF littermates. In addition, these results reveal that a further reduction in residual wild-type Cftr mRNA levels in this model does not influence the severity of the histopathological reaction to S. aurus, which is a critical threshold for functional CFTR.

Source link: https://doi.org/10.1159/000076470


Residual cftr Expression Varies with Age in cftr tm1Hgu Cystic Fibrosis Mice: Impact on Morphology and Physiology

In most strains, mouse models for cystic fibrosis mimic intestinal manifestations of the disease, but lung disease phenotypes are lacking. Cftr mRNA in renal and respiratory epithelia varied with age from Ztm:MF1 controls at the age of 2 and 4 months to those in patients with CFTR splice mutations beyond the age of 6 months. Compared to Ztm:MF1 controls, the alveolar surfactant phospholipid pool was increased in cftr tm1Hgu mice by 1. 5- to 2-fold. u2013 was reduced in cftr tm1Hgu mice, relative to littermate controls, in obsteolar clearance measurements. Although no clear lung pathology was evident in the cftr expression of cftr tm1Hgu mice, surfactant phospholipid homeostasis, and alcociliary clearance were abnormal, alpha and mucociliary clearance were not abnormal.

Source link: https://doi.org/10.1159/000067308


Office-Based Sinus Surgery for Cystic Fibrosis Chronic Rhinosinusitis

Objective: Chronic rhinosinusitis is almost common in the cystic fibrosis population, and many patients require multiple endoscopic sinus surgeries throughout their lifetime. Recent studies have shown the significant pulmonary and systemic health benefits of comprehensive CRS therapy, as well as the numerous human and systemic health benefits. This research seeks to determine the safety and possibility of doing revision endoscopic sinus surgery for CF patients in the outpatient hospital setting using only local anesthesia to minimize the need for mechanical ventilation and inpatient hospitalization. Methods: This is a prospective cohort study carried out at a tertiary care academic medical center with a CF Foundation-accredited care center. This prospective pilot study is the first to demonstrate the safety and effectiveness of performing comprehensive revision endoscopic sinus surgery for CF patients in an outpatient clinic setting using only local anesthesia.

Source link: https://doi.org/10.1159/000512495

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions

* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions